OEIS Complex: A Case Report

Abstract

OEIS complex describes a combination of birth defects, including omphalocele, extrophy of the bladder, imperforate anus, and spinal defects. OEIS complex involves abnormalities of almost every body system and shows variability from case to case. The etiology of OEIS complex is thought to be multifactorial. The prenatal diagnosis of OEIS complex is difficult; however, the diagnosis of OEIS mainly relies on sonographic findings. The major sonographic findings include nonvisualization of the bladder, persistent cloaca, omphalocele, and myelomeningocele. Significantly increased maternal serum alphafetoprotein is also reported. Surgical intervention has improved the survival of patients born with OEIS complex; however, the success of these procedures cannot guarantee a good quality of life. The survivors may suffer with long-term psychosexual developmental difficulties.

Complejo OEIS (onfalocele, extrofia vesical, ano imperforado y defectos espinales), una entidad más confusa de lo que se cree. Reporte de caso

“…Most OEIS complex cases are isolated and caused by a multifactorial alteration involving environmental factors such as smoking and exposure to benzodiazepines during pregnancy (17) and genetic factors such as deletion of 9q 34.1-q, 1p36 and 3q12.2-3q I1.2, trisomy 18, mosaic Turner syndrome, mutations in mitochondrial 125rRNA and mutations in homeobox genes such as HLXB9 since recurrence has been reported in some families with monozygotic conjoined twins (2,(18)(19)(20). Almost all cases are premature with low birth weight; however, there are reports of patients with an average gestational age of 37.5 weeks and proper weight, height, and head circumference for age (21).…”

A neonate with Omphalocele-Extrophy-Imperforate anus-Spinal defects (OEIS) complex

“…Correction is occurs in stages, which involves closure of omphalocele, separation of ileocecal connection from the exstrophied bladder plate and reapproximating the end colostomy with preservation of all bowels. Even though survival has been improved greatly due to advancement of surgical techniques, prognosis is variable which depends on the magnitude of the associated anomalies 3 . However, long term complications like bladder and bowel incontinence can result in reduced quality of life 3 .…”

“…It occurs as a result of defective closure of anterior abdominal wall and maldevelopment of cloaca and urogenital septum due to defect in blastogenesis. It is estimated to affect 1: 200,000 to 400,000 pregnancies 1 .Even though it has been reported in siblings 2 , its occurrence is believed to be sporadic 3 . The prognosis is variable 3 .…”

“…It is estimated to affect 1: 200,000 to 400,000 pregnancies 1 .Even though it has been reported in siblings 2 , its occurrence is believed to be sporadic 3 . The prognosis is variable 3 .…”